CAMEO
EOSINOPHILIC CELLULITIS (WELLS' SYNDROME) C.L. GOH, M.D.
A 25-year-old Indian presented with an acute episode of indolent localized swellings of 2 days duration on his left forearm. He had recurrent localized indolent swelling of 1 year duration on his face, arms, and legs. Each attack lasted about 1 week and cleared spontaneously. He had experienced about seven to eight attacks over the 1 year. There was no known precipitating factor. Each attack was not preceded by symptoms of infection. He did not take any medication prior to his attack. There was no significant past history. There was no personal or family history of atopy. Clinical examination revealed nonerythematous, localized, indurated, mildly tender, nonpitting swellings on his left forearm. There was no systemic abnormality. His blood counts revealed a hemoglobin of 14.8 g%, total white count of 8500/mm^ with a differential count of 68% polymorphs, 15% lymphocytes, 2% monocytes, and an eosinophilia of 15%. His platelet count was 300,000/mm3. Peripheral blood film was normal. His antimicrofilaria antibody test was negative. His serum immunoglobulin levels, liver function test, and renal function test were normal. Urine microscopic examination was normal. Skin biopsy (Fig. 1) showed presence of dermal edema. There were superficial and deep perivascular and interstitial infiltrates of eosinophils throughout the dermis. The eosinophilic infiltrate extended right into the subcutaneous fat. Areas of necrosis of collagen with abundant eosinophils were present. Eosinophils were present in abundance within the lumina of the vessels. The changes was consistent with eosinophilic cellulitis. The patient's skin lesion cleared within 2 days after starting on oral prednisolone 30 mg daily. He continued to experience recurrent attacks at 4 to 6 monthly intervals, each attack was aborted with a short course of oral prednisolone. He remained in good health otherwise when he was reviewed after 1 year.
Figure 1. Low power photomicrograph showing inflammatory changes throughout the dermis consisting of leukocytic infiltrate predominantly of eosinophils. There are large areas of dermal fibrinoid degeneration, necrosis, and dense infiltrates between collagen bundles (flaming figures). the English dermatology literature.'"''' We are not aware of any report of eosinophilic cellulitis in Asians. Eosinophilic cellulitis (Wells' syndrome) is an uncommon dermatosis, which usually presents with recurrent localized swelling on the limbs. Recent cases presenting with acute onset of unusual urticarial eruptions or infiltrated erythemas and vesiculopapular eruption'"''^ have been reported. The presentation can be varied. In our patient, because of his dark skin color, there was no observable erythema. His presentation was that of recurrent asymptomatic indolent indurated swelling. The pathogenesis of the condition has been reported to represent a hypersensitivity reaction. It has been reported to be triggered off by several factors including arthropod bites, parasitic infestation, malignancy, and drugs. "'''* This condition usually responds rapidly to oral steroids but may take several weeks to clear.'""'' Unlike other reports, in each attack
DISCUSSION
Eosinophilic cellulitis is an acute inflammatory cutaneous disease with distinctive histopathologic findings of dermal edema and eosinophilic infiltrates in early lesions, and histiocytes surrounding collagenous masses in later stages. The condition was first described by Wells in 1971 in four patients who presented with recurrent localized swelling of the extremities.' It is a rare disorder. There have been less than 50 cases reported in From the National Skin Centre, Singapore.
" ..
Address for correspondence: C.L. Goh, M.D., National Skin Center, 1 Mandalay Road, Singapore 1130. . . 429
International Journal of Dermatology Vol. 31, No. 6, June 1992
the skin eruptions in our patient cleared rapidly without scarring within a few days of instituting prednisolone therapy. It appears that the severity of the skin eruptions and response to treatment in eosinophilic cellulitis is variable. Eosinophilic cellulitis may be confused with cellulitis or urticarial vasculitis, but can be easily confirmed by skin biopsy. The lesion usually manifests initially as a burning sensation of itching with redness and local induration. The lesion may occasionally be annular or circinate in appearance. Occasionally local atrophy similar to morphea'^ follows, but this usually resolves without treatment. Eosinophilia is usually present''"' as is seen in our patient. Associated bone marrow eosinophilia has also been reported previously.^-''"'' There are usually no associated immunologic abnormalities. The histologic features of eosinophilic cellulitis are characteristic. There is usually marked dermal edema and infiltration of the dermis with eosinophils, which also accumulate around vessels; eosinophilic infiltrates intersperse between collagen bundles producing "flame figure"^'' in older lesions as seen in our patient's biopsy. This case report in an Asian adds to the few case reports of this distinct entity. In a dark-skinned patient the skin lesion may be indiscernible, as was the case in our patient, because erythema is absent. It may present as recurrent indolent cutaneous swellings. It is important for dermatologists to be aware of this disease (which can often be confirmed by a skin biopsy) as it often responds readily to steroid therapy.
Spigel GT, Winkelman RK. Wells' syndrome: recurrent granulomatous dermatitis with eosinophilia. Arch Dermatol 1979; 115:611-613. Wells GC, Smith NP. Eosinophilic cellulitis. Br | Dermatol 1979; 100:101-109. Nielsen T, Schmidt H, Sogaard H. Eosinophilic cellulitis (Wells' syndrome) in a child. Arch Dermatol 1981; 177:427-429. Varotti C, Tosti A, Gohbi M, ct al. Eosinophilic cellulitis. A new case. Dermatologica 1982; 164:404-406. Bonvalet D, Caron C, Levet R, et al. Le syndromede Wells ou cellulite a eosinophiles. Ann Dermatol Venereol 1983; 110:899-907. , • Peters MS, Schroeter AL, Gleich GJ. Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells' syndrome. Br J Dermatol 1983; 109:141-148. Stern JB, Sobel HJ, Rotchford JP. Wells' syndrome: is there collagen damage in the flame figures.' J Cutan Pathol 1984; 11:501-505. 9.
Dijkstra JWE, Bergfeld WF, Steck WD, et al. Eosinophilic cellulitis associated with urticaria. A report of 2 cases. J Am Acad Dermatol 1986; 14:32-38.
10.
Lindskov R, Ilium N, Weismann K, Thomsen OF. Eosinophilic cellulitis: five cases. Acta Derm Venereol (Stockh) 1988; 68:325-330.
11.
Clark DP, Anderson PC. Eosinophilic cellulitis caused by arthropod bites. IntJ Dermatol 1988; 27:411-412.
12.
Newton JA, Greaves MW. Eosinophilic cellulitis (Well's Syndrome) with florid histological changes. Clin Exp Dermatol 1988; 13:318-320.
13.
Andreano JM, Kantor GR, Bergeld W, et al. Eosinophilic cellulitis and eosinophilic pustular folliculitis. | Am Acad Dermatol 1989; 20:934-936.
14.
Panizzon R. Wells' syndrome (eosinophilic cellulitis): additional cases in the literature. J Am Acad Dermatol 1989; 20:1136-1137.
REEERENCES
1.
Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 1971; 57:46-56.
PERFUMED CASTOR OIL.
From the collection of "La Pharmacie Fran^aise," New Orleans, Louisiana, Mr. Ben Bavly, Curator. 430
EOSINOPHILIC CELLULITIS (WELLS' SYNDROME) C.L. GOH, M.D.
A 25-year-old Indian presented with an acute episode of indolent localized swellings of 2 days duration on his left forearm. He had recurrent localized indolent swelling of 1 year duration on his face, arms, and legs. Each attack lasted about 1 week and cleared spontaneously. He had experienced about seven to eight attacks over the 1 year. There was no known precipitating factor. Each attack was not preceded by symptoms of infection. He did not take any medication prior to his attack. There was no significant past history. There was no personal or family history of atopy. Clinical examination revealed nonerythematous, localized, indurated, mildly tender, nonpitting swellings on his left forearm. There was no systemic abnormality. His blood counts revealed a hemoglobin of 14.8 g%, total white count of 8500/mm^ with a differential count of 68% polymorphs, 15% lymphocytes, 2% monocytes, and an eosinophilia of 15%. His platelet count was 300,000/mm3. Peripheral blood film was normal. His antimicrofilaria antibody test was negative. His serum immunoglobulin levels, liver function test, and renal function test were normal. Urine microscopic examination was normal. Skin biopsy (Fig. 1) showed presence of dermal edema. There were superficial and deep perivascular and interstitial infiltrates of eosinophils throughout the dermis. The eosinophilic infiltrate extended right into the subcutaneous fat. Areas of necrosis of collagen with abundant eosinophils were present. Eosinophils were present in abundance within the lumina of the vessels. The changes was consistent with eosinophilic cellulitis. The patient's skin lesion cleared within 2 days after starting on oral prednisolone 30 mg daily. He continued to experience recurrent attacks at 4 to 6 monthly intervals, each attack was aborted with a short course of oral prednisolone. He remained in good health otherwise when he was reviewed after 1 year.
Figure 1. Low power photomicrograph showing inflammatory changes throughout the dermis consisting of leukocytic infiltrate predominantly of eosinophils. There are large areas of dermal fibrinoid degeneration, necrosis, and dense infiltrates between collagen bundles (flaming figures). the English dermatology literature.'"''' We are not aware of any report of eosinophilic cellulitis in Asians. Eosinophilic cellulitis (Wells' syndrome) is an uncommon dermatosis, which usually presents with recurrent localized swelling on the limbs. Recent cases presenting with acute onset of unusual urticarial eruptions or infiltrated erythemas and vesiculopapular eruption'"''^ have been reported. The presentation can be varied. In our patient, because of his dark skin color, there was no observable erythema. His presentation was that of recurrent asymptomatic indolent indurated swelling. The pathogenesis of the condition has been reported to represent a hypersensitivity reaction. It has been reported to be triggered off by several factors including arthropod bites, parasitic infestation, malignancy, and drugs. "'''* This condition usually responds rapidly to oral steroids but may take several weeks to clear.'""'' Unlike other reports, in each attack
DISCUSSION
Eosinophilic cellulitis is an acute inflammatory cutaneous disease with distinctive histopathologic findings of dermal edema and eosinophilic infiltrates in early lesions, and histiocytes surrounding collagenous masses in later stages. The condition was first described by Wells in 1971 in four patients who presented with recurrent localized swelling of the extremities.' It is a rare disorder. There have been less than 50 cases reported in From the National Skin Centre, Singapore.
" ..
Address for correspondence: C.L. Goh, M.D., National Skin Center, 1 Mandalay Road, Singapore 1130. . . 429
International Journal of Dermatology Vol. 31, No. 6, June 1992
the skin eruptions in our patient cleared rapidly without scarring within a few days of instituting prednisolone therapy. It appears that the severity of the skin eruptions and response to treatment in eosinophilic cellulitis is variable. Eosinophilic cellulitis may be confused with cellulitis or urticarial vasculitis, but can be easily confirmed by skin biopsy. The lesion usually manifests initially as a burning sensation of itching with redness and local induration. The lesion may occasionally be annular or circinate in appearance. Occasionally local atrophy similar to morphea'^ follows, but this usually resolves without treatment. Eosinophilia is usually present''"' as is seen in our patient. Associated bone marrow eosinophilia has also been reported previously.^-''"'' There are usually no associated immunologic abnormalities. The histologic features of eosinophilic cellulitis are characteristic. There is usually marked dermal edema and infiltration of the dermis with eosinophils, which also accumulate around vessels; eosinophilic infiltrates intersperse between collagen bundles producing "flame figure"^'' in older lesions as seen in our patient's biopsy. This case report in an Asian adds to the few case reports of this distinct entity. In a dark-skinned patient the skin lesion may be indiscernible, as was the case in our patient, because erythema is absent. It may present as recurrent indolent cutaneous swellings. It is important for dermatologists to be aware of this disease (which can often be confirmed by a skin biopsy) as it often responds readily to steroid therapy.
Spigel GT, Winkelman RK. Wells' syndrome: recurrent granulomatous dermatitis with eosinophilia. Arch Dermatol 1979; 115:611-613. Wells GC, Smith NP. Eosinophilic cellulitis. Br | Dermatol 1979; 100:101-109. Nielsen T, Schmidt H, Sogaard H. Eosinophilic cellulitis (Wells' syndrome) in a child. Arch Dermatol 1981; 177:427-429. Varotti C, Tosti A, Gohbi M, ct al. Eosinophilic cellulitis. A new case. Dermatologica 1982; 164:404-406. Bonvalet D, Caron C, Levet R, et al. Le syndromede Wells ou cellulite a eosinophiles. Ann Dermatol Venereol 1983; 110:899-907. , • Peters MS, Schroeter AL, Gleich GJ. Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells' syndrome. Br J Dermatol 1983; 109:141-148. Stern JB, Sobel HJ, Rotchford JP. Wells' syndrome: is there collagen damage in the flame figures.' J Cutan Pathol 1984; 11:501-505. 9.
Dijkstra JWE, Bergfeld WF, Steck WD, et al. Eosinophilic cellulitis associated with urticaria. A report of 2 cases. J Am Acad Dermatol 1986; 14:32-38.
10.
Lindskov R, Ilium N, Weismann K, Thomsen OF. Eosinophilic cellulitis: five cases. Acta Derm Venereol (Stockh) 1988; 68:325-330.
11.
Clark DP, Anderson PC. Eosinophilic cellulitis caused by arthropod bites. IntJ Dermatol 1988; 27:411-412.
12.
Newton JA, Greaves MW. Eosinophilic cellulitis (Well's Syndrome) with florid histological changes. Clin Exp Dermatol 1988; 13:318-320.
13.
Andreano JM, Kantor GR, Bergeld W, et al. Eosinophilic cellulitis and eosinophilic pustular folliculitis. | Am Acad Dermatol 1989; 20:934-936.
14.
Panizzon R. Wells' syndrome (eosinophilic cellulitis): additional cases in the literature. J Am Acad Dermatol 1989; 20:1136-1137.
REEERENCES
1.
Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 1971; 57:46-56.
PERFUMED CASTOR OIL.
From the collection of "La Pharmacie Fran^aise," New Orleans, Louisiana, Mr. Ben Bavly, Curator. 430
